Respiratory Syncytial Viral Infection in an Infant with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly in children that requires necessary and urgent repair. We report a child who was hospitalized with respiratory failure due respiratory syncytial viral (RSV) infection and was subsequently diagnosed with ALCAPA. Aggressive treatment for RSV included synagis and nebulized ribavirin prior to surgical repair. After waiting 4 weeks for the RSV infection to resolve, she underwent successful left coronary artery reimplantation on hospital day 27 and has regained normal left ventricular size and function.     

Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventriclular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.     

Anatomic Repair of Left Main Coronary Artery Atresia: Coronary Ostioplasty With Autologous Pulmonary Artery

BackgroundLeft main coronary arterial (LMCA) atresia is a rare coronary arterial anomaly with extremely limited data on the optimal management. We aimed to report our single-surgeon experience of the ostioplasty in patients with LMCA atresia.MethodsFrom July 2018 to December 2019, pediatric patients who presented with LMCA atresia and subsequently underwent surgical coronary ostioplasty were recruited into this retrospective study. Concomitant mitral repair was applied when the regurgitation was moderate or more severe.ResultsA total of 9 patients diagnosed with LMCA atresia were included. Mitral regurgitation was found in all of them, including 6 (66.7%) severe, 1 (11.1%) moderate, and 2 (22.2%) mild. In addition to ischemic lesions, which were found in 7 (77.8%) patients, structural mitral problems were also common (presented in 7 [77.8%] patients). All the patients underwent coronary ostioplasty with autologous pulmonary arterial patch augmenting the anterior wall of the neo-ostium. Mean aortic cross clamp time and cardiopulmonary bypass time was 88.1 ± 18.9 and 124.6 ± 23.6 minutes, respectively. During a median of 10.9 (range: 3.3 to 17.2) months' follow-up, there was only 1 death at 5 months after surgery. All survivors were recovered uneventfully with normal left-ventricular function; however, with 4 (50.0%) having significant recurrence of mitral regurgitation.ConclusionsWith favourable surgical outcomes, coronary ostioplasty for LMCA atresia may be an option of revascularization. Structural mitral problems presented in majority patients, resulting in the requirement of concomitant mitral repair. However, the optimal technique of mitral repair remains unclear.     

Repair of an Anomalous Left Coronary Artery from the Pulmonary Artery in a Premature Neonate

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. It is typically diagnosed at a few months of age. The infant presents with cardiac failure secondary to left ventricular dysfunction from the coronary steal that develops as pulmonary vascular resistance (PVR) falls. With the diagnosis made, surgical repair is typically performed expeditiously in order to try and restore left ventricular perfusion and stop the ongoing coronary steal. We present an unusual case of a preterm infant undergoing a routine echocardiogram, who was incidentally found to have an ALCAPA. Management strategies between neonatology and cardiology may differ in this setting. In this case, elevated PVR helps to preserve myocardial perfusion prior to surgical repair. Therefore, common neonatal management strategies typically utilized for respiratory distress, elevated PVR, and cardiac dysfunction may have potential for detrimental effects on myocardial perfusion in this specific lesion. This case also emphasizes the importance of identifying the coronary origins as a routine part of a complete pediatric echocardiogram. It also presents a rarely encountered problem: when to repair an ALCAPA in an as yet asymptomatic patient. We believe this to be the youngest and smallest patient reported with the diagnosis and successful surgical correction of an ALCAPA.     

Myocardial Flow Reserve in Long-Term Survivors of Repair of Anomalous Left Coronary Artery From Pulmonary Artery

Objectives. This study sought to evaluate regional myocardial flow reserve in long-term survivors of repair of anomalous left coronary artery from pulmonary artery (ALCAPA) and to relate the flow abnormalities to the patients’ exercise performance.Background. Patients with ALCAPA usually present during infancy with severe ischemic cardiomyopathy. The left ventricular function recovers after surgical repair. However, the extent of recovery of myocardial blood flow (MBF) and its potential physiologic significance in long-term survivors are unknown.Methods. We evaluated MBF (ml/g per min) at baseline and during maximal coronary vasodilation by adenosine in 11 patients after ALCAPA repair (median age 17 years, range 7 to 22) using nitrogen-13 ammonia and dynamic positron emission tomographic imaging. Patients also underwent an incremental exercise test with metabolic monitoring. In each patient, MBF was quantified in the three major vascular territories: the left anterior descending and left circumflex coronary artery territories and the right coronary artery (control region) territory.Results. Basal MBF was mildly reduced in the left coronary territories versus the control region (0.79 ± 0.14 vs. 0.85 ± 0.19, p = 0.05). During hyperemia, flow in the left coronary territories was significantly lower than that in the control region (2.1 ± 0.5 vs. 2.6 ± 0.5, p < 0.001). As a result, myocardial flow reserve was lower in the left coronary territories than in the control region (2.6 ± 0.7 vs. 3.2 ± 0.7, p < 0.001). Exercise performance was impaired in patients when compared with age-matched control subjects. Maximal oxygen consumption correlated linearly with maximal hyperemic flows in the left coronary artery territories (r = 0.73, p = 0.03).Conclusions. Long-term survivors of ALCAPA repair demonstrate regional impairment of myocardial flow reserve. This may contribute to impaired exercise performance by limiting cardiac output reserve.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

An Unusual Combination of an Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) and a Right Coronary Artery System with Two Separate Ostia from the Aorta in an Adult

We describe a patient with an infrequent combination of variants in both the right and the left coronary arterial ostia, namely a combination of two separate right coronary artery (RCA) ostia from the aorta, and an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). To our knowledge, such a combination has not been previously reported. Based on published statistics for individual variants, such a combination is expected to occur approximately once for every 500,000 to one million live births. ALCAPA and dual RCA anatomy was characterized in our patient by echocardiography, conventional angiography, and multidetector computed tomography before and after Takeuchi repair. (Echocardiography 2010;27:E13-E17)     

应用再植技术治疗起源异常左冠状动脉的效果分析

目的:分析和讨论应用冠状动脉再植技术治疗先天性起源于肺动脉的左冠状动脉异常(ALCAPA)的外科治疗效果和经验。方法:回顾分析2008年4月~2013年4月26例应用冠状动脉再植技术治疗先天性ALCAPA患儿的临床资料。其中男14例,女12例;年龄4月~6岁。术前均经超声心动图,CT和或心血管造影检查明确诊断。结果:术前左室射血分数(LVEF)20~80(48±18)%,心胸比0.54~0.77(0.64±0.07)。并发二尖瓣关闭不全(MI)轻度以上21例,其中中度以上17例。左心室心尖部室壁瘤1例,房间隔缺损1例。同期行二尖瓣成形11例,室壁瘤切除1例,房间隔缺损修补1例。本组患儿手术均顺利完成,无手术死亡。体外循环时间91~238(150±37)min,心肌阻断时间64~200(126±36)min。随访1~61(29±19)月,患儿无远期死亡。最后一次随诊LVEF35~75(64±11)%,心胸比0.48~0.65(0.57±0.05),较术前明显改善。MI中度以下24例。结论:冠状动脉再植技术手术效果良好,对于中度以上MI应积极治疗。     

Unique Echocardiographic Markers of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) in the Adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect in adults. We report a 38‐year‐old male presenting with exertional syncope. He was referred for the evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Echocardiography revealed mild left ventricular enlargement, abnormal flow‐pattern in the ventricular septum and dilatation of the right coronary artery. Pulsed‐wave Doppler with sample volume placed in the coronary ostium showed systolic coronary flow predominancy. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. Coronary angiography confirmed ALCAPA syndrome. Surgical correction was planned.     

The Management of the Older Adult Patient with Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Presentation of Two Cases and Review of the Literature

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.     

Repair of anomalous origin of left coronary artery from the pulmonary artery

Patients with anomalous origin of the left coronary artery from the pulmonary artery often have mitral valve regurgitation. Although establishing dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled. Between April 1999 and August 2005, 8 patients underwent surgical correction at our institution. There were 4 males and 4 females, aged from 9 months to 13 years (mean, 6.4 years). Six patients underwent direct aortic reimplantation and 2 had a Takeuchi procedure. Simultaneous mitral annuloplasty was performed in 7 patients with moderate or severe mitral regurgitation. There were no deaths or postoperative complications. Follow-up ranged from 4 to 80 months (mean, 34 +/- 26 months). Left ventricular function improved significantly from a preoperative fractional shortening of 0.21 +/- 0.09 to 0.35 +/- 0.06. Mitral regurgitation decreased on follow-up in the 7 patients who had mitral annuloplasty. We recommend performing mitral annuloplasty at the time of operation in patients with moderate or severe mitral regurgitation and anomalous origin of the left coronary artery from the pulmonary artery.     

Adult‐Type Anomalous Origin of the Left Coronary Artery from the Main Pulmonary Artery: One Case Report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect that presents even less frequently in adults. Here we described a 40‐year‐old patient presenting with palpitations. Electrocardiography revealed frequent ventricular ectopy. Echocardiography revealed a dilated left ventricle and an abnormal flow pattern in the pulmonary artery and at the right side of the interventricular septum. Coronary angiography demonstrated an enlarged right coronary artery (RCA) with collateralization to the left coronary artery (LCA) and reflux of contrast into the pulmonary artery. Computed tomography confirmed ALCAPA syndrome. Surgical corrections were planned. (ECHOCARDIOGRAPHY, Volume **, ***********)     

Left ventricular geometry and operative mortality in patients undergoing mitral valve replacement.

BACKGROUND: Distortion of left ventricular (LV) shape is often associated with LV dysfunction and is thought to be an independent predictor of survival in patients with coronary disease. HYPOTHESIS: The purpose of this study was to examine the relationship between LV geometry and hospital mortality in patients with mitral regurgitation (MR) undergoing mitral valve surgery. METHODS: A consecutive series of patients (aged 68+/-12 years, 47% men) (n = 149) with MR who underwent cardiac catheterization, left ventriculography, and mitral valve surgery from 1995 to 1996 at Mount Sinai Medical Center was studied. Left ventriculograms, clinical records, and hemodynamics were reviewed. Left ventricular volumes and ejection fraction were calculated using standard techniques. Left ventricular shape in diastole and systole was evaluated using the sphericity index, which is defined as the end-systolic LV volume (x 100) divided by the volume of a sphere whose diameter is equal to the LV long axis. RESULTS: In the patients studied, the etiology of mitral insufficiency was mitral valve prolapse in 40.9%, ischemic heart disease in 40.3%, rheumatic heart disease in 11.4%, and prosthetic valvular dysfunction in 7.4%. The average ejection fraction was 65%+/-17. Systolic sphericity index (SSI) was 36%+/-15 in patients who died, compared with 25%+/-11 in patients who lived (p < 0.001). A multivariate model was constructed using hemodynamic and angiographic indices derived during preoperative cardiac catheterization. Systolic sphericity index (odds ratio = 1.6 for each point increase, p < 0.01) was found to be an independent predictor of postoperative survival in the global population, as well as in patients with coronary disease (p<0.01). CONCLUSION: Left ventricular geometry is an independent angiographic risk factor for survival following mitral valve replacement. Sphericity index is a simple method for assessing LV geometry which should be calculated in patients as part of risk stratification.     

Incidental Discovery of Anomalous Left Coronary Artery Arising from the Pulmonary Artery in a Coronavirus Disease-2019 Patient: A Blessing in Disguise

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a serious congenital malformation. Reports about asymptomatic, incidentally discovered ALCAPA in adults are scarce. We describe a patient with no known pre-existing cardiac condition admitted to our hospital with coronavirus disease 2019 (COVID-19) and was incidentally found to have ALCAPA. To the best of our knowledge, this is the first reported case of incidentally discovered ALCAPA in a COVID-19 patient and highlights the importance of appropriate investigation of the coronary status by Multidetector Cardiac Computed Tomographic Angiography (MDCCTA) in individuals with asymptomatic left ventricular dysfunction. The presentation of this case, discussion and literature review serves to iterate the necessity of appropriately investigating patients with asymptomatic LV dysfunction.     

An Unusual Presentation of an Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) in an Adult: Anterior Papillary Muscle Rupture Causing Severe Mitral Regurgitation

We describe a 29-year-old male, previously in good health, with no history of angina pectoris and no risk factors for ischemic heart disease presenting with biventricular failure and severe mitral valve regurgitation. There were no signs or serological test results to suggest infective endocarditis. Transthoracic echocardiography (TTE) revealed severe anterior mitral valve prolapse secondary to papillary muscle rupture, severe mitral valve regurgitation, as well as an anterior myocardial wall hypokinesis. Parasternal short-axis view showed an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), which was confirmed on coronary angiography. This is an unusual presentation of ALCAPA in an adult.     

右冠状动脉病变对左冠状动脉狭窄患者左心室功能的影响

目的　探讨右冠状动脉病变对左冠状动脉狭窄患者左心室功能的影响及其机制。方法　对比分析左冠状动脉狭窄患者在合并与不合并右冠状动脉病变时的左心室射血分数。结果　与相应部位单纯左冠状动脉狭窄患者相比 ,合并右冠状动脉病变患者左心室射血分数均呈不同程度地下降 ,其中在左前降支、左前降支 +左回旋支狭窄基础上合并右冠状动脉病变时左心室射血分数下降有统计学意义 (P <0 .0 5或 0 .0 1) ,左主干合并右冠状动脉狭窄患者下降幅度最大 ,但无统计学意义。结论　右冠状动脉病变可在单纯左冠状动脉狭窄的基础上使左心室收缩功能进一步恶化 ;当左冠状动脉狭窄部位为左前降支、左主干或左前降支 +左回旋支时 ,对左心室收缩功能影响更为严重     

Coronary echocardiography in tetralogy of fallot: diagnostic accuracy, resource utilization and surgical implications over 13 years

OBJECTIVES: This study sought to determine the diagnostic accuracy and impact of the systematic use of coronary echocardiography in a large group of preoperative patients with tetralogy of Fallot (TOF). BACKGROUND: Accurate preoperative identification of an anomalous coronary artery crossing the right ventricular outflow tract (RVOT) in patients with TOF is important to prevent coronary injury during surgical repair. METHODS: A retrospective review identified 598 patients with TOF between 1983 to 1995 who underwent an echocardiogram at <2 years old before complete surgical repair. Associated diagnoses included pulmonary stenosis (n = 433), pulmonary atresia (n = 121), common atrioventricular canal (n = 17), absent pulmonary valve syndrome (n = 24) and aortopulmonary window (n = 3). RESULTS: Based on intraoperative findings, 32 patients (5.4%) were found to have a major coronary artery crossing the RVOT. The use and diagnostic performance of coronary echocardiography increased over time, while the number of patients undergoing preoperative cardiac catheterization declined. During the most recent study period (1991 to 1995, n = 274), 97% of patients underwent coronary echocardiography yielding a sensitivity of 82%, specificity of 99% and accuracy of 98.5%. Of the 18 patients with TOF and pulmonary stenosis who had abnormal coronary arteries during this period, only 6 (33%) required an extracardiac conduit as part of their complete repair. CONCLUSIONS: Coronary echocardiography is an accurate noninvasive tool to delineate coronary anatomy in infants with TOF before complete repair. Routine preoperative cardiac catheterization solely for diagnosis of coronary anatomy is not necessary. The use of an extracardiac conduit can be avoided in the majority of patients with TOF and pulmonary stenosis who have a major coronary artery crossing the RVOT.     

彩色多普勒检测肺静脉血流评价冠心病患者左心室舒张功能

目的　探讨彩色多普勒超声心动图检测冠心病患者肺静脉血流频谱评估左心室舒张功能的价值。方法　冠心病组 4 0例 ,无心脏疾患者 (对照组 ) 30例 ,用彩色多普勒超声心动图检测各组肺静脉血流频谱(PVEP)、二尖瓣血流频谱 (MVFP)的变化和左室舒张功能的关系。结果　与对照组比较 ,冠心病组PVEP中S峰速度加快 ,D峰速度减慢 ,S/D >1,AR波明显加快 ,持续时间延长 ,两组之间有明显差异 (P <0 0 0 1) ;MVFP中E峰降低 ,A峰增加 ,E峰减速时间 (EDT)延长 ,E/A <1。结论　肺静脉血流频谱结合二尖瓣血流频谱能较全面评价冠心病患者的左心室舒张功能。     

Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery Presenting Following Relief of Left Heart Obstruction: A Distinct and Predictable Clinico-Pathological Syndrome

Introduction. Pre-operative recognition of significant abnormalities of the coronary arteries is important in a variety of congenital cardiac conditions. Failure to diagnose anomalous origin of the coronary artery from the pulmonary artery during repair of other anomalies is important because reduction in pulmonary artery pressure will reduce myocardial perfusion pressure. Patients. We report two cases of the rare association of anomalous origin of the left coronary artery from the right pulmonary artery, aortic coarctation, and mitral stenosis. Conclusions. Definitive imaging of coronary artery anatomy by echocardiography or other modalities should form a routine part of diagnostic assessment in all congenital heart disease patients but particularly those with left heart obstruction.     

Left ventricular function in cyanotic congenital heart disease

Left ventricular function was studied with quantitative biplane cinean-giocardiography in 39 preoperative and 23 postoperative patients wlth cyanotic congenital heart disease. Diagnoses included pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (group 1), tricuspld atresia (group 2) and pulmonary atresia with ventricular septal defect (group 3). Preoperative patients ranged in age from 1 day to 7 years and postoperative patients from 7 weeks to 23 years. Left ventricular end-diastolic volume was increased in preoperative patients in groups 1 and 2 (132 and 136 percent of normal, respectively) but was normal in patients in group 3. Left ventricular ejection fraction was decreased to a similar extent in preoperative groups 1 to 3: 0.54, 0.55 and 0.56, respectively. After a shunt procedure left ventricular end-diastolic volume increased to 228 and 266 percent of normal in groups 1 and 2, respectively, but remained within normal limits in group 3. Left ventricular ejection fraction was normal in postoperative group 1 patients, whose ages averaged 1.8 years, but remained decreased in group 2 and 3 patients, whose ages averaged 8.1 and 5.6 years, respectively. Duration of cyanosis and degree of left ventricular dilatation appear to be important variables in regard to pump function in patients with cyanotic congenital heart disease.